Immunology 1A: Autoimmune and auto-inflammatory diseases 2 Flashcards

1
Q

Which antibodies do systemic diseases produce?

A

ANA

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2
Q

Graves’ disease pathophysiology

A

IgG antibodies to TSH receptor –> uncontrolled production of thyroid hormones which cannot be overrided by negative feedback

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3
Q

What kind of hypersensitivity is Grave’s?

A

Type II

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4
Q

Which thyroiditis is associated with a high T4 and which with low?

A

High; Viral thyroiditis (de quervain’s)

Low: Hashimoto’s thyroiditis

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5
Q

clinical features of hashimoto’s

A

Constipation, lethargy cold intolerance etc

GOITRE - irregular

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6
Q

Autoantibody in Hashimoto’s

A

anti-TPO and anti-thyroglobulin

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7
Q

Why is it not useful to measure autoantibodies in Hashimotos?

A

They are present in many women who do not exhibit any symptoms

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8
Q

Hypersensitivtiy type of Hashimoto’s

A

Type II and IV

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9
Q

Pathophysiology of T1DM? Therefore what hypersensitivity is it?

A

CD8+ T cells recognise autoantigens on pancreatic B cells

Type IV-mediated

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10
Q

On 1st presentation of T1DM, what else do you screen for?

A

Coeliac’s, TFTs and LFTs

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11
Q

What autoantigens are recognised by T cells in T1DM?

A

GAD and IA2

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12
Q

Antibodies seen in T1DM (often predate symptoms but not used diagnostically)

A

Anti-GAD
Anti-islet cell
anti-insulin
anti-IA2

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13
Q

Clinical fx of B12 deficiency

A

Subacute degeneration of cord
Optic neuropahty
Peripheral neuropathy

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14
Q

Pernicious anaemia autoantibodies

A

Autoantibodies to gastric parietal cells and intrinsic factor

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15
Q

Myasthenia gravis autoantibodies

A

Autoantibodies to ACh receptor

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16
Q

Special test for diagnosis

A

Tensilon test

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17
Q

MG type of hypersensitivity?

A

Type II

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18
Q

Offending antibody in Goodpasture’s

A

antibody vs alpha3 subunit of type IV collagen

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19
Q

Immunohistochemistry for anti-GBM, what is a +ve result?

A

Linear IgG deposition along basement membrane

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20
Q

What is seen on biopsy in GBM?

A

Cresentic nephritis

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21
Q

Goodpasture’s hypersensitivity

A

Type II

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22
Q

Rheumatoid arthritis HLA ASSOCIATIONS

A

HLADR4/HLADR1 (Bind to citrullinated peptides with higher affinity)

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23
Q

Which is the most SPECIFIC autoantibody in RA?

A

Anti-CCP

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24
Q

2 GENETIC predispositions to RA?

A

HLAdR4/DR1

PAD2 + 4 polymorphisms (deamination of arginine to citrulline)

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25
Q

What infection is associated with RA and why?

A

Porphyromonas gingivalis - expresses PAD enzyme thus promoting citrullination

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26
Q

Why does smoking increase RA risk?

A

Erosion and increased citrullination

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27
Q

What is rheumatoid factor

A

IgM antibody targeted against Fc region of IgG antibody

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28
Q

B cell involvement in rheumatoid arthritis - give 2 ways

A

Type II - Antibodies bind to citrullinated peptides –> complement activation, macrophage activation and NK cell activation with ADCC

Type III response - immune complex formation by anti-CCP and anti-RF + deposited –> complement activation

29
Q

How is the joint affected macroscopically in RA?

A

Increased synovial fluid
Inflamed synovial tissue –> pannus
Pannus invades articular cartilage and adjacent bone

30
Q

4 Diseases associtaed with ANA +ve

A

SLE
Sjogrens
Systemic sclerosis
Dermato/polymyositis

31
Q

Which cells are used to test for aNAs?

A

Stain Hep2 cells

32
Q

Pathogenesis of SLE

A

Antibodies against intracellular proteins

IMMUNE COMPLEXES formed + deposited in tissues

Complement activation + activation of cells expressing Fc receptors
(type III hypersensitivity)

33
Q

Is ANA specific to autoimmune diseases?

A

No, present in normal population

34
Q

How does the staining pattern / immunofluorescence differ in SLE and Goodpasture’s?

A
Goodpasture's = linear, smooth
SLE = Lumpy bumpy due to immune complex deposition
35
Q

Which antigens involved in SLE?

A

DNA, histones and RNP

36
Q

You request an anti nuclear antibody test on two patients with joint pain
Patient A’s result is 1:640
Patient B’s result is 1:80
Based on this information, which has the “strongest” (i.e most positive) antibody?

A

Patient A’s

37
Q

4 ribonuclear ANAs

A

Ro,La, Sm, U1RNP

38
Q

Which autoantibody test is highly specific for SLE?

A

Anti-dsDNA

39
Q

anti-ENA vs anti-dsDNA antibodies

Which one is useful in monitoring disease activity, and in which disease?

A

Anti-dsDNA in SLE

40
Q

homogenous staining of the nucleus is associated with..?

A

anti-dsDNA

41
Q

speckled staining of the nucleus is associated with antibodies to…?

A

ENA (Ro,La,Sm, U1RNP)

42
Q

aNTI-ro, ANTI-LA?

A

Sjorgens

43
Q

Antibodies in diffuse CREST

A

Anti-topoisomerase (Anti-Scl70)

44
Q

Antibodies in limited CREST

A

Anti-centromere

45
Q

What other investigation can be done in SLE?

A

Measure complement

46
Q

Which complement factor depleted first in SLE?

A

C4 first then C3

47
Q

C3 and C4 levels in inactive, moderate and very active lupus

A

inactive = normal C3 + C4
Moderate = low C4, normal C3
Severe/v active = Low C4 ADN C3

48
Q

Antiphospholipid syndrome antibodies

A

Anti-cardiolipin
Lupus anticoagulant
Anti-B2 glycoprotein 1

49
Q

Symptoms of APLS

A

Clots, obstetric complications, livedo reticularis recurrent venous or arterial thrombosis

50
Q

Thickening around the mouth, microstomia

A

Systemic sclerosis

51
Q

What does CREST stand for?

A
Calcinosis
Raynaud's
Eosophageal dysmotility
Sclerodactyly
Telangiectasia
52
Q

Diffuse cutaneous scleroderma symptoms

A

CREST + more severe GIT involvement + interstitial pulmonary disease + renal problems

53
Q

Differentiating between diffuse and limited cutaneous scleroderma (CREST)

A

Diffuse: skin involvement progresses BEYOND the forearms (Does not progress in CREST, involved PERI-ORAL SKIN)

54
Q

Immunofluorescence findings in diffuse cutaneous scleroderma

A

Nucleolar pattern

55
Q

Antibodies found in diffuse cutaneous scleroderma

A

Anti-topoisomerase (Scl70), RNA pol I,II,III and Fibrillarin

56
Q

Antibodies found in CREST syndrome

A

Anti-centromere

57
Q

As well as CREST, what other symptom is found in CREST syndrome?

A

primary pulmonary HTN

58
Q

weakness, malaise, periorbital heliotropic rash, Gottron’s papules

A

Dermatomyositis

59
Q

Dermatomyositis clinical features

A

Periorbital Heliotrope rash + Gottron’s papules

60
Q

Dermatomyositis hypersensitivity type

A

Type 3, immune complex mediated vasculitis

61
Q

Polymyositis hypersensitivity type

A

Type 4 - CD8 cells kill myofibers via perforin/granzymes

62
Q

Antibodies in myositis

A

Dermatomyositis + polymyositis: Anti-Jo-1
DM >PM Anti-Mi-2
PM - Anti-SRP

63
Q

3 types of small vessel associated ANCA vasculitis

A

Granulomatosis with polyangiitis (GPA - Wegner’s)
Eosinophilic granulomatosis with polyangiitis (Churg Strauss)
microscopic polyangiitis

64
Q

cANCA antibodies to…

A

Proteinase 3

65
Q

pANCE antibodies to…

A

Myeloperoxidase

66
Q

Staining in cANCA and pANCA

A

cANCA - cytoplasmic fluorescence

pANCA - perinuclear staining pattern

67
Q

pANCA - which diseases?

A

MPA and eGPA

68
Q

What is ANA used for?

A

Connective tissue disease screening

69
Q

What is ANCA used for?

A

Small vessel vasculitis including MPA, GPA, eGPA